IgG4-related Lung Disease with Organizing Pneumonia Effectively Treated with Azathioprine

Yasutaka Onishi; Tetsuji Kawamura; Ryogo Kagami; Yasuharu Nakahara; Mochiduki Yoshiro

doi:10.2169/internalmedicine.53.2564

CASE REPORTS

IgG4-related Lung Disease with Organizing Pneumonia Effectively Treated with Azathioprine

Yasutaka Onishi, Tetsuji Kawamura, Ryogo Kagami, Yasuharu Nakahara, Mochiduki Yoshiro

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Keywords: IgG4-related lung disease, IgG4-related pulmonary disease, IgG4-related disease, organizing pneumonia, azathioprine, prednisolone

JOURNAL OPEN ACCESS

2014 Volume 53 Issue 23 Pages 2701-2704

DOI https://doi.org/10.2169/internalmedicine.53.2564

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Abstract

Immunoglobulin (Ig)-G4-related disease is a multi-organ disease that may affect the lung. We herein describe a patient with IgG4-related lung disease (IgG4-RLD) who was radiologically and pathologically diagnosed with organizing pneumonia. He was successfully treated with a combination of prednisolone (PSL) and azathioprine (AZA), and his clinical course has been uneventful since tapering off PSL. This is a rare case of IgG4-RLD manifesting as organizing pneumonia, and, to our knowledge, this is also the first case showing the effectiveness of AZA in treating IgG4-RLD.

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